Improvement in Patient-reported Hearing After Treatment With Bevacizumab in People With Neurofibromatosis Type 2.

Objective: Assess patient-reported outcomes (PRO) for hearing and tinnitus relative to clinical hearing assessment in people with neurofibromatosis 2 (NF2) associated hearing loss.

Study Design: Prospective, open label, phase-II clinical trial with PRO administered pre-, post-, and after treatment.

Setting: Three tertiary referral centers.

Efficacy and Biomarker Study of Bevacizumab for Hearing Loss Resulting From Neurofibromatosis Type 2-Associated Vestibular Schwannomas.

Purpose: Neurofibromatosis type 2 (NF2) is a tumor predisposition syndrome characterized by bilateral vestibular schwannomas (VSs) resulting in deafness and brainstem compression. This study evaluated efficacy and biomarkers of bevacizumab activity for NF2-associated progressive and symptomatic VSs.

Patients And Methods: Bevacizumab 7.

Bevacizumab for progressive vestibular schwannoma in neurofibromatosis type 2: a retrospective review of 31 patients.

Objective: Early studies suggest that bevacizumab treatment can result in tumor shrinkage and hearing improvement for some patients with neurofibromatosis type 2 (NF2). The aim of this study was to report extended follow-up in a larger cohort of similarly treated patients.

Study Design: Retrospective study.

Audiology in the sudden hearing loss clinical trial.

Objective: To report the pretreatment and posttreatment population characteristics and the overall stability of the audiologic outcomes found during the Sudden Hearing Loss Clinical Trial (ClinicalTrials.gov: Identifier NCT00097448).

Study Design: Multicenter, prospective randomized noninferiority trial of oral versus intratympanic (IT) steroid treatment of sudden sensorineural hearing loss (SSNHL).

Improvement in word recognition score with level is associated with hearing aid ownership among patients with hearing loss.

Market surveys consistently show that only 22% of those with hearing loss own hearing aids. This is often ascribed to cosmetics, but is it possible that patients apply a different auditory criterion than do audiologists and manufacturers? We tabulated hearing aid ownership in a survey of 1000 consecutive patients. We separated hearing loss cases, with one cohort in which word recognition in quiet could improve with gain (vs.

Erlotinib for progressive vestibular schwannoma in neurofibromatosis 2 patients.

Objective: In vitro treatment of Nf2-deficient cells with epidermal growth factor receptor (EGFR) inhibitors can reduce cellular proliferation. We sought to determine the activity of erlotinib for progressive vestibular schwannoma (VS) associated with neurofibromatosis 2 (NF2).

Study Design: Retrospective case review.

Hearing improvement after bevacizumab in patients with neurofibromatosis type 2.

Background: Profound hearing loss is a serious complication of neurofibromatosis type 2, a genetic condition associated with bilateral vestibular schwannomas, benign tumors that arise from the eighth cranial nerve. There is no medical treatment for such tumors.

Methods: We determined the expression pattern of vascular endothelial growth factor (VEGF) and three of its receptors, VEGFR-2, neuropilin-1, and neuropilin-2, in paraffin-embedded samples from 21 vestibular schwannomas associated with neurofibromatosis type 2 and from 22 sporadic schwannomas.

Suggested response criteria for phase II antitumor drug studies for neurofibromatosis type 2 related vestibular schwannoma.

Neurofibromatosis type 2 (NF2) is a tumor suppressor gene syndrome characterized by multiple schwannomas, especially vestibular schwannomas (VS), and meningiomas. Anticancer drug trials are now being explored, but there are no standardized endpoints in NF2. We review the challenges of NF2 clinical trials and suggest possible response criteria for use in initial phase II studies.

Hearing aids and cochlear damage: the case against fitting the pure tone audiogram.

Herein we propose a different approach to hearing aids, an approach that flows logically from the pathophysiology of cochlear disorders. This approach challenges some central tenets of the industry by 1) suggesting that many, if not most, cases would be better served by flat, undistorted gain across all frequencies rather than by "fitting" gain to the audiogram; and 2) suggesting that most of the improvements in hearing aid technology are targeted at reducing patient complaints as opposed to increasing measurable word recognition performance. We recommend that it is better to accommodate the damaged cochlea in these cases than to attempt to reverse-engineer the audiometric test results.

Clinical implications of a damaged cochlea: pure tone thresholds vs information-carrying capacity.

The pure tone audiogram is an accurate measure of auditory threshold as a function of stimulus frequency. However, it does not provide the complete picture in patients with sensorineural hearing loss (SNHL) because it is not a direct measure of damage to the cochlear epithelium or of the associated limits on information-carrying capacity that restrict word recognition. Diagnostic use of the audiogram leads to the error of viewing SNHL as "dB of hearing loss," which may seem reversible by gain.

Twenty years of audiology in a patient with Norrie disease.

Objective: To describe disease progression and treatment outcomes over a 20-year period (ages 5-25) in a young man with Norrie disease (occuloacousticocerebral dysplasia), ND; OMIM #310600. Affected individuals are born blind and develop progressive sensory loss with onset in adolescence. This disease is X-linked and has been associated with mutations of the NDP gene (Xp11.

Audiologic and radiographic response of NF2-related vestibular schwannoma to erlotinib therapy.

Background: A 48-year-old man presented to a neurologist with complaints of bilateral hearing loss and tinnitus. The patient was a member of a large family affected by neurofibromatosis type 2 and first noted hearing loss 10 years before presentation.

Investigations: Medical and neurological examination, MRI scan of the brain and spinal cord, pure-tone audiometry, NU-6 monosyllabic word test with phoneme scoring, City University of New York topic-related sentences test, noise/voice test of minimal auditory capability battery.

Incidence of responses at the lower audiometric limits.

Purpose: To put forward a symbol extension, consisting of an upward arrow added to current symbols, to denote that a patient continued to respond at the audiometric limit (i.e., -10 dB HL).

Using audiometric thresholds and word recognition in a treatment study.

Objectives: First, to examine a possible limit on significant results imposed by a progressive floor effect for hearing threshold improvement in a treatment study. This floor effect for hearing recovery suggests that if inclusion criteria are not set sufficiently high, the superiority of a treatment group may not be detectable. Second, to examine the outcomes when using two different types of criteria for significant change in a subject's word recognition score.

Audiologic features of Norrie disease.

Objectives: Norrie disease is an X-linked recessive disorder in which patients are born blind and develop sensory hearing loss in adolescence. The hearing loss associated with Norrie disease has been shown in a genetically altered knockout mouse to involve dysfunction of the stria vascularis; most other structures are preserved until the later stages of the disease. The objective of this study was to characterize the audiologic phenotype of Norrie disease for comparison with the pathophysiologic mechanism.

Oral steroid treatment of sudden sensorineural hearing loss: a ten year retrospective analysis.

Objective: To describe ten years of experience with Sudden Sensorineural Hearing Loss and compare the outcomes with and without treatment with oral corticosteroids.

Study Design: Retrospective review of medical records.

Setting: Large specialty hospital, Department of Otolaryngology.

The tuning curve in clinical audiology.

This article will explore the audiograms formed by the expected psychophysical thresholds from single, normally functioning inner hair cells. Like the audiogram formed by the vibrotactile response region, these psychophysical tuning curves represent fundamental limits in audiometry since they are the worst possible thresholds expected, even if no other cells are functioning. These examples can be put to many uses, but the most important lesson of the hypothetical tuning curve audiogram is that whereas each cell gives rise to thresholds across many frequencies, it cannot be expected to transmit more than one cell's worth of speech information.

Vascular defects and sensorineural deafness in a mouse model of Norrie disease.

Norrie disease is an X-linked recessive syndrome of blindness, deafness, and mental retardation. A knock-out mouse model with an Ndp gene disruption was studied. We examined the hearing phenotype, including audiological, histological, and vascular evaluations.